28 Aug 00 - CJD -Human carriers of CJD may help spread disease

The possibility that apparently healthy people and animals could be carriers of BSE and variant CJD is raised today by a Government adviser.

Research by a team in London, led by Prof John Collinge, may lead to a re-think of the scale of the BSE epidemic in cattle, and could mean that key experiments into how easily BSE can move from cattle to infect people and other animals have to be repeated.

mad cow disease, BSE, is caused by prions, infectious proteins that also cause fatal brain diseases such as Creutzfeldt-Jakob disease in humans and scrapie in sheep. The Medical Research Council Prion Unit reports in the Proceedings of the National Academy of Sciences on new evidence for the existence of a "sub-clinical" form of BSE - a symptom-free infection - which was unknown until now.

Many species, such as sheep, pigs and poultry, were exposed to BSE via contaminated feed, meaning it was possible that they might also harbour the newly-discovered sub-clinical infection , said Prof Collinge, a member of the Government's Spongiform Encephalopathy Advisory Committee, in the report.

The measures to protect people against BSE were adequate but the implications should be thought through, he said. He said: "We should re-think how we measure species barriers in the laboratory. We should not assume that just because one species appears resistant to a strain of prions that they do not silently carry the infection. This raises the possibility that apparently healthy cattle could harbour, but never show signs of, BSE."

He said that if there was a silent form of variant CJD in humans they could put others at risk through blood transfusions or contaminated surgical instruments . The silent form was a concern because prions from symptom-free mice in Prof Collinge's experiments were still able to kill other mice and animals . In other words, sub-clinical BSE could lead to a reservoir of silent disease that could infect and kill people.

The findings could also overturn estimates of the prevalence of BSE, said Prof Collinge. It might, for example, explain why only one or two animals in a herd exposed to BSE succumbed - the others might have had a silent form of BSE. Prof Collinge said: "These new findings have important implications for those responsible for preventing infected material getting into the food chain and for those considering how best to safeguard health."

The team examined the species barrier, which limits the ability of prions to jump from one species to infect another; such infections between cattle and humans caused variant CJD in the UK and France. The researchers found that mice could develop a sub-clinical form of spongiform disease in which they carried high levels of infectivity but did not develop the clinical disease during their normal lifespan, suggesting that the incubation period is much longer than the lifespan.

The height of the species barrier varies widely between different combinations of animals and with the type or strain of prions. While some barriers are quite small - for instance, BSE easily infects mice - other combinations of strain and species show a seemingly impenetrable barrier. Until now, the particular barrier studied was assumed to be robust, but the experiments might have to be repeated, said the professor.

The Department of Health welcomed the work and has asked SEAC to consider the findings at its next meeting on Sept 29, but it stressed that current measures to protect public health were introduced on the basis that infection in animals and in people might be present in the absence of clinical disease.

Prof Peter Smith, acting chairman of SEAC, said: "We will discuss the implications of this research paper at the next meeting. The findings are of considerable interest and further our knowledge about prion-related diseases. It is not clear that the new findings indicate that additional controls should be considered with respect to protecting human or animal health, but this will be considered along with consideration of what further research may be necessary.

"The controls recommended by SEAC with respect to the protection of both human and animal health have always been on the basis that there were likely to be undetected cases of infection in both the cattle and human populations. These recommendations have been adopted by the Government."

Alarming findings by scientists investigating mad cow disease has raised new fears over the safety of beef and other meats sold in butchers and supermarkets.

They suggest that BSE may jump from one species to another more easily than was previously supposed without showing any symptoms.

In a new paper the scientists, led by world renowned expert Professor John Collinge, say there could be "important public health implications". Not only cattle, but sheep , pigs and poultry exposed to BSE via animal feed may have developed a "subclinical" form of the disease which remained symptom-free and hidden .

Animals thought to be healthy and incapable of acquiring BSE could theoretically pass the disease on to humans . The possibility of further action to protect public health is now to be examined by a body of experts advising the Government on mad cow disease.

Members of the Spongiform Encephalopathy Advisory Committee (SEAC) are to consider the findings at their next meeting on September 29, where they will question Professor Collinge.

Professor Peter Smith, acting chairman of SEAC, said: "It is not clear that the new findings indicate that additional controls should be considered with respect to protecting human or animal health, but this is an issue that will be considered at our next meeting, along with consideration of what further research may be necessary as a consequence of the findings."

The research is published in the journal Proceedings of the National Academy of Sciences. Prof Collinge's team from the Medical Research Council Prion Unit based at St Mary's Hospital, west London, took a closer look at the "species barrier" which most experts believe makes it difficult for BSE-type diseases to spread between different species of animal.

The findings suggest this may be a mistaken assumption made because of the lack of symptoms from a previously unknown form of subclinical BSE , which leaves an animal looking healthy.

Variant CJD, which first emerged in 1996, is now known to be BSE in a human guise. It is thought to have spread to the human population through people eating contaminated beef products in the 1980s. A total of 79 cases of variant CJD have been recorded so far.

A spokesman for the Ministry of Agriculture, Fisheries and Food, said: "We believe the safeguards we have in place at the moment are adequate to deal with the issues Prof Collinge raises, but of course we will listen to what he has to say."

28 Aug 00 - CJD - Cattle may have BSE without symptoms for years

By Steve Connor, Science Editor

Independent ... Monday 28 August 2000

Government advisers on "mad cow" disease are to investigate fresh evidence that cattle can be badly infected with bovine spongiform encephalopathy (BSE) for years without showing any symptoms .

New research has shown that certain strains of prions, the infectious agents thought to cause BSE, can infect laboratory mice yet the animals never develop the disease .

The findings indicate that BSE can exist in a "sub-clinical" form, where high levels of infectious agent are present in an animal yet fail to result in any symptoms. The same may also be true of variant Creutzfeldt Jakob Disease, vCJD, the human equivalent of BSE.

Sub-clinical disease is different from the incubation period for BSE, when relatively low levels of the prion agent begin to build up and spread within an infected animal, eventually to cause symptoms.

Professor Peter Smith, the acting chairman of the Spongiform Encephalopathy Advisory Committee, said the latest findings, published in the Proceedings of the National Academy of Sciences, are of "considerable interest" and will be discussed in detail when it meets at the end of next month.

The research, by scientists at the Medical Research Council's prion unit, based at St Mary's Hospital in London, demonstrated that mice remain healthy when infected with large amounts of prions from infected hamsters.

Previous research had shown that mice never develop the disease when injected with hamster prions, leading scientists to believe that there was a large "species barrier" protecting mice from this source of infection.

Furthermore, when infective material from the symptomless mice was subsequently injected into other mice, these animals did develop symptoms , suggesting that a new strain of prion had been created.

John Collinge, the leader of the research team, said that the results show that just because a species appears resistant to BSE, it does not mean that they are free of infection . "This research raises the possibility, which has been mentioned before, that apparently healthy cattle could harbour, but never show signs of, BSE ," he said.

Professor Collinge said current measures to protect the public from BSE did not need to be changed, but he wanted to see a national system of testing brains of cattle and other species exposed to BSE for signs of prion infection .

27 Aug 00 - CJD - Damning report on BSE adds to Tory woe

By Colin Brown, Political Editor

Independent ... Sunday 27 August 2000

Tory leader William Hague is preparing for a fresh blow to the Conservatives' election hopes with the publication of the long-awaited report into the BSE scandal .

Lord Phillips, who chaired the two-year long investigation, is planning to deliver his findings to the Government by the end of next month, say sources close to the inquiry. And one member of Mr Hague's shadow cabinet admitted that the report would be highly critical of the previous Tory government : "We are going to have to take it on the chin. There's nothing else we can do," he said.

Conservative Party sources are calculating that the main criticism will be directed away from Mr Hague's existing front-line team. "Angela Browning is the only one who is in the shadow cabinet now and she won't take the blame," said one Conservative source.

Mr Hague, who saw his hopes of a Tory revival dashed in the opinion polls last week, was Welsh Secretary at the height of the BSE controversy and a member of John Major's Cabinet.

The Phillips investigation uncovered a trail of incompetency under the last Tory administration that allowed potentially dangerous offal to get into the food chain after it had been banned.

Lord Phillips is expected to highlight the buck-passing which was evident in the public hearings as ex-ministers and officials sought to shift the blame to local authorities for allowing lax controls on slaughter houses.

The line-up of top former Tory ministers who were questioned by the inquiry included former agriculture ministers, John MacGregor, William Waldegrave, John Gummer and Douglas Hogg.

The handling of the inquiry has angered some former Tory ministers. "There was information on the inquiry website that was inaccurate that had to be completely rewritten. The way it was handled was appalling," said one ex-minister.

The Phillips inquiry has warned Commons officials that the report will be "the size of packing case " when it is published in October after the Commons returns from the summer break.

Mr Hague, who is certain to face renewed speculation about his leadership unless the Tories narrow Labour's lead soon, is about to launch his own ballot of the party to support key policy proposals including rejecting the euro, £5 per week on the state pension and cutting taxes on business. But there are fears of a low turn-out.

The Tory leader this week will call for more protection for children in care and measures to end "political correctness" in adoption, creating a national register and removing county boundaries.

John Prescott, the Deputy Prime Minister, revealed Labour ministers are counting on the BSE inquiry to remind voters of the "total incompetence of Tory government ".

Mr Prescott, who has been running the Government's day-to-day operations while Mr Blair has been on holiday, ridiculed the Tory summer offensive. Speaking to the Independent on Sunday, the Deputy Prime Minister said the failure of the Tories to press home their attacks and the embarrassment caused by Mr Hague's claim to drink 14 pints of beer a day as a teenager will have unnerved the Conservatives.

"Whoever organised the Tory summer campaign must have been drinking 14 pints a day. I'd be happy to pay their drinks bill. The Tory offensive was based on fear and prejudice over asylum seekers, paedophiles and now travellers but it has been a total flop," said Mr Prescott.

He said: "Our people got a few jolts about how the Tories play all these fear and prejudice issues. It must be the first example of the failure of negative campaigning in Britain.

"I think now our people are fired up for the election. A lot of people were nervous and apprehensive about the Tory attacks. But it was the best thing they could have done to us.

"Gordon Brown has delivered on public spending. Our supporters just want to knock the hell out of the Tories."

21 Aug 00 - CJD - Beef acid trials aid weight loss

Staff and agencies

Guardian ... Monday 21 August 2000

Patient trials show that the fat-fighting properties of a popular supplement for slimmers do work. Studies in the United States and Norway revealed that conjugated linoleic acid (CLA) helped the overweight shed fat, and improved the control of adult-onset diabetes.

CLA is a fatty acid occurring in many dairy products and beef (UK Correspondents note: beef derivatives have gone out of fashion since BSE/CJD .....). Studies on animals suggest it also enhances immune function, and combats several types of cancer and narrowing of the arteries.

Results of the first human trials of the supplement were presented at the annual meeting of the American Chemical Society in Washington.

Eighty overweight people, in a study at the University of Wisconsin, Madison, dieted and exercised. Most initially lost weight and then regained it on stopping their diets. But those taking CLA regained less fat and retained more muscle. Results from Norway suggested CLA might aid weight loss even without dieting.

At Purdue University, Indiana, an eight-week diabetes study with 22 volunteers showed that, of those taking CLA, 64% showed an improvement in insulin levels. Martha Belury, a chief researcher, said coupling CLA with pharmaceuticals could cut the health care costs of type-two diabetes and the side effects of long-term drug use.

13 Aug 00 - CJD - Knacker's yard link to CJD cluster

Jonathan Leake and Dipesh Gadher

Sunday Times ... Sunday 13 August 2000

Britain's worst outbreak of the human form of mad-cow disease may be linked to a nearby knacker's yard that sold meat from diseased animals . The yard operated just eight miles from Queniborough, the Leicestershire village where health officials are investigating the first known cluster of CJD cases.

Three people who spent time in the village died from CJD in 1998, and a fourth person is suspected of having the degenerative brain disease. Another victim lived just three miles away.

The possible link to the knacker's yard - which recycled animals unfit for human consumption into pet food and other products - dates back 20 years , to about the time when scientists now believe the BSE epidemic may have begun.

Two meat traders from Bedfordshire were convicted in 1982 of buying unapproved beef from W E Mason & Sons of Wigston, near Leicester, and selling it to an unsuspecting butcher in Hertfordshire.

Last week officials seized council documents and court reports relating to the company to determine whether any unfit meat may have entered the human food chain locally.

"We have had a very useful series of conversations about this with Oadby and Wigston council," said Philip Monk, a consultant in communicable disease control at Leicestershire health authority, who is heading the Queniborough investigation. "I am ruling nothing in and nothing out. Anything we have that is potentially helpful in explaining local meat trading practices has to be examined."

The case heard by Leicester magistrates in 1982 was the culmination of Operation Meat Hook, a joint investigation between detectives and environmental health officers from three counties.

The teams covertly observed Peter Fletcher, a partner in a wholesale butcher's business near Dunstable, on four occasions in 1980 when he visited Leonard Mason, the yard's owner. He loaded beef carcasses from the yard into an un-marked van, which had been contaminated by a cow's head "fouled by stomach contents", according to evidence given in court. One of the carcasses was later found to have been infected with pleurisy.

Fletcher marked the meat with a fake inspector's stamp, and then left it with a retail butcher near Hemel Hempstead, Hertfordshire.

"A knacker's yard may, and frequently will, deal with diseased cattle," the prosecutor had told an earlier hearing. "Meat may be partly decomposed and contaminated. Disease is rife in such premises and could include anthrax and tuberculosis."

Fletcher was jailed for three months and fined £500. His partner, Francis Fensome, received a suspended prison sentence. Mason was cleared after telling the court that he had been told the meat was to be used to feed animals at Whipsnade zoo.

The knacker's yard, which had been run by the Mason family since 1947, was closed the same year and now stands derelict. Mason has since died.

Last week his brother, Jack Mason, said: "I am confident there is no connection with us and the outbreak in Queniborough. Most of the meat went to zoos. Any meat that was sold locally went to dog owners as pet food."

There is no proof that Mason dealt in cattle infected with BSE, which was not recognised at the time. But such yards commonly dealt in "downer " cows - those displaying symptoms of illness - so any animals that did have BSE were likely to have ended up in such places.

The Queniborough inquiry team is also examining slaughtering techniques at Leicestershire abattoirs and childhood eating habits of those who grew up in the village, although school meals have been ruled out as a possible cause of the CJD outbreak.

Arthur Beyless lost his daughter, Pamela , 24, a bank worker, to the disease after a two-year struggle for survival. Although the Beylesses live in nearby Glenfield, Pamela regularly visited her grandparents in Queniborough and the family often bought meat from Ian Bramley, the village butcher, in the late 1970s and early 1980s.

Beyless said: "On one occasion I was buying some meat when Ian told me he'd got it for 'a good deal'. It does make you wonder when you consider this theory about the knacker's yard. This disease is something that might never have happened if people weren't always grasping for that last penny."

The other two named victims with links to Queniborough are Stacey Robinson , 19, who lived there for 12 years before moving to another part of the county, and Glen Day , 34, who worked on a farm in the area. He regularly ate at the Horse and Groom pub, which was supplied with meat by Bramley.

Bramley died in a car crash. His stepmother, Hazel Bramley, said she knew nothing about Mason's yard. "We bought our meat directly from local farmers," she said.

"The animals were slaughtered in Leicester and delivered to us. I don't know anything about this place in Wigston."

10 Aug 00 - CJD - Victims of CJD fewer than feared

Tim Radford, science editor

Guardian ... Thursday 10 August 2000

Variant CJD, the human form of mad cow disease, could ultimately claim 136,000 lives, according to Oxford scientists.

But this figure is the worst case scenario, say statisticians who have been trying to map the impact of the disease on the British population. They report in Nature today that this will be the number of deaths if the incubation period for the disease is 60 years or more.

When the first cases of vCJD shocked ministers in 1996 - they had repeatedly claimed that the mysterious cattle disease could not be a danger to humans - there seemed no limit to the numbers potentially at risk from infected beef. A European committee conjectured that a single beast with BSE could put 500,000 people at risk .

But Neil Ferguson, Azra Ghani, Christl Donnelly and Roy Anderson at the Wellcome Trust centre for epidemiology at Oxford University began looking at the pattern of cases and the ages of the victims, the percentage of the population most at risk, the relative infectivity of cattle at the incubation stage and the effectiveness of control measures. They calculated up to 5m scenarios and then matched them against the cases of the disease in humans.

Any sick animal that got into the food market was liable to infect no more than two individuals, and only 40% of the population was genetically susceptible. That left one key factor in the calculation - the mean incubation period of the disease itself. If the incubation period was less than 20 years, then there could be as few as 63 cases. If the incubation period stretched almost to lifetime length, then there were likely to be 136,000 . What mattered over the next few years was the annual rate of cases.

"If the average annual incidence of vCJD over the next three years is fewer than 15 cases, then the maximum total number of cases would fall to 20,000," they write.

Classical or sporadic CJD is a disease of the old. But the victims of vCJD are all relatively young. The statisticians were anxious to see if the average ages of the cases increased with time. If they did, then it would mean that everybody was equally susceptible but younger people had shorter incubation periods. They now rule out that possibility.

"It is something about being young that makes you vulnerable ," said Dr Ghani. "Either vulnerable or exposed to a greater extent. It could be just the types of food that people eat, but it is impossible in this sort of statistics to distinguish between the two. The upper limit to the epidemic scenario has decreased quite substantially but it is still very large."

10 Aug 00 - CJD - CJD: the news improves, but the mystery remains

Science Editor Tim Radford

Guardian ... Thursday 10 August 2000

The latest research reveals how little we really know about Creutzfeldt-Jakob disease (CJD)

The air is full of good news about the dreadful new disease, variant CJD (vCJD). There are now likely to be no more than 136,000 deaths in a worse-case scenario, down from the 500,000 previously estimated by a European committee set up to study its causes, and there could be a good deal fewer.

It all depends on the "mean incubation period" for the disease, according to the Wellcome Trust Centre for Epidemiology of Infectious Diseases at Oxford University in a letter to Nature magazine.

However, this statement exposes how little we know about CJD. Would the "incubation period" be less than 20 years for most people? If so, the number of deaths will be dramatically fewer than even the new estimate.

Or is it on average 60 years or more, the period on which the revised estimate is based? If so, we can expect it to claim more victims, although perhaps not as many as were thought a year ago.

The original version of disease itself is for the most part a riddle: roughly one in a million cases, anywhere in the world, vegetarians or meat eaters. It was, however, a disease of that appeared to be restricted to the elderly.

The new variant - the one linked with bovine spongiform encephalopathy (BSE) and thought to be contracted by eating part of an animal contaminated with BSE, or "mad cow" disease - is however, so far a disease of the young.

That too, raises a number of questions: How is it contracted? Is it something to do with being young? Or is it contacted from what the young eat? How indeed, did British cows get it?

The cows may have consumed baked and rendered infected animal carcasses. But if the disease is spread by eating, then its agent has to survive cooking, digestion, and then travel from the stomach to the brain.

How does it do that? How much infected beef needs to be consumed to contract the disease? And how many infected cattle have entered the food chain since the disease began? Which parts of the beast are the most infectious? When controls were first imposed, how well were they observed?

BSE first appeared in about 1986: for a while, British government ministers tried to pretend it posed no threat . Indeed, pet-food manufacturers were the first to act, banning the use of the cattle's eyes, brains, spleens, tonsils and so on in their products a year before the government banned them in food earmarked for human consumption, in 1990.

Zoo animals such as kudu, oryx, ocelot and elk which that fed infected food began to contract BSE, but the government still protested that the disease could not cross a species barrier to harm humans.

Only in 1996, with the first horrifying evidence of a new kind of CJD very like the bovine version, did the government act. But by then, an epidemic was inevitable .

The statisticians at Oxford are doing what they can: They look at the pattern of events, construct possible scenarios and categorise them by in order of probability. Next year they will know a bit more, and with each ensuing year they will have clearer projections of what lies ahead.

With luck, each year the expected toll will fall. But that still won't be good news: There is no cure, no vaccine and no way for consumers to protect against its contraction. It is a wretched way to die.

But however many cases are to come, vCJD will always seem like the disease that need never have happened .

07 Aug 00 - CJD - Study to 'rule out' BSE link to milk

Keith Perry

Guardian ... Monday 7 August 2000

The government has ordered an inquiry into whether there is a link between BSE and milk and dairy products .

The research, announced by Nick Brown, the minister of agriculture, follows assurances that dairy products are safe from causing variant CJD, the human form of BSE.

The food standards agency has asked scientists to carry out a three-year study into possible links "as soon as possible". They are also being told to re-examine previous studies which have given milk and dairy products the all-clear.

A spokeswoman for the Ministry of Agriculture said that the inquiry would cost £800,000. It followed a recommendation by the spongiform encephalopathy advisory committee (Seac ), set up by the government to monitor the brain-wasting disease.

"Milk is safe but this research is to put it beyond doubt," the spokeswoman said. "It is a precautionary measure and previous studies have shown milk is safe." (UK Correspondents note: Government does not spend £800k for nothing, there is strong evidence that that calves have become infected with BE from their mother's milk ).

CJD deaths have so far been blamed on eating meat from infected cattle. But some scientists are concerned that earlier studies are unreliable, and that the infective agent could be passed on through milk .

Fourteen Britons died from CJD in the first six months of this year - as many as in the whole of 1999. Since 1995 it has claimed 69 lives and Seac said that the incidence of the CJD was increasing by between 20 and 30% a year .

Last week it was reported that there have been 76 definite and probable cases of CJD in Britain, including seven possible victims still alive.

Last month the government launched an inquiry into a cluster of CJD deaths around the village of Queniborough in Leicestershire. Three of the four victims died within weeks of each other . Robert Will, head of the government's CJD surveillance unit, said at the time that baby food and school meals may have been a source of the village outbreak.

Gill Turner of the CJD Support Network welcomed news of the three-year research. "CJD is still a poorly understood disease and we welcome any research and money that is going to be invested into it.

"The concern is not knowing whether there is a risk from cow's milk. Athough there has been no evidence up to now that you can get CJD from milk, further study has to be a good thing."

Milk safety has been questioned since an interim UK study suggested that BSE could be transmitted from cow to calf. The report raised fears about possible BSE infection through milk and led to some German states blocking imports of British dairy products.

05 Aug 00 - CJD - Mother who died may have had CJD

By Helen Rumbelow, Medical Reporter

Times ... Saturday 5 August 2000

A young mother is believed to have been the latest victim of the human form of "mad cow" disease, an inquest has been told. Anita Maria Bradshaw, 30, died last week after suffering two years of a mysterious illness, which the preliminary pathologist's report indicates was variant Creutzfeldt- Jakob disease (vCJD).

Timothy Dawson, who specialises in the brain and spinal cord, said that he believed Mrs Bradshaw had the disease , but the inquest, at Blackburn, will be adjourned until December for the results of further tests.

Mrs Bradshaw's husband, Andrew, said at his home in Accrington, Lancashire, that it had been agonising watching his wife suffer for so long.

"It seemed to start off with post-natal depression after the birth of our son, Reece, and after that she was in and out of hospital all the time. I don't really know what she was suffering from and I won't find out for sure until the full inquest in December. All I know is that she was very, very ill ."

The number of deaths from the disease has more than quadrupled since 1995, bringing them to 69 confirmed fatalities, according to new research.

04 Aug 00 - CJD - CJD deaths increasing by a third each year

By Steve Connor, Science Editor

Independent ... Friday 4 August 2000

Cases of the human version of BSE have risen markedly for the first time , according to an analysis published today in the medical journal The Lancet.

Latest figures show that the number of deaths from the human form of bovine spongiform encephalopathy is increasing by one-third each year , with a total number of 79 definite and probable cases up to the middle of July.

Although the number of new cases of variant Creutzfeldt-Jakob disease (vCJD) have increased steadily since 1994, the figures are now large enough to reveal a statistically significant rise .

"We believe that our findings reflect a real increase in the incidence of vCJD in the UK. Such an increase is clearly a matter of concern, although we emphasise that the absolute number of cases is low," say the scientists from the Public Health Laboratory Service, the London School of Hygiene and Tropical Medicine and the National CJD Surveillance Unit.

The study analysed the date of onset of the brain-wasting disease as well as deaths between 1994 and 2000. The number of onsets increased by 23 per cent each year, whereas deaths increased by 33 per cent between 1995 and 2000. The underlying incidence has risen from an estimated two new cases per quarter in 1994 to 6.5 new cases this year .

Hester Ward, consultant epidemiologist at the CJD Surveillance Unit, said that 14 people died from vCJD in the first six months of this year. The figures point to between 25 and 30 new cases by the end of the year, compared with 17 for the whole of 1999.

"This is the first time this analysis has reached statistical significance . We really can't tell the size of the epidemic until we've reached the peak, all we can say is that it is increasing but we don't know for how long," Dr Ward said. She said it was premature to say the worst was over . "I don't think you can say that from these figures. The worst is not over for this year."

04 Aug 00 - CJD - CJD deaths are rising by a third every year

By David Derbyshire, Medical Correspondent

Telegraph ... Friday 4 August 2000

Deaths from the human form of BSE have risen by a third every year since the disease was first detected, according to a new study.

While statisticians stress that numbers are still too small to make meaningful predictions, they say there is some evidence of a rising trend in variant Creutzfeldt- Jakob disease cases over the past six years.

The findings come from a breakdown of the official figures compiled since 1996. The researchers, led by scientists at the Government's CJD surveillance unit in Edinburgh, also found that new cases of vCJD had risen by an average of 23 per cent a year.

Dr Hester Ward, one of the Edinburgh researchers, said: "Although absolute numbers remain low, there appears to be a real increase in the incidence of vCJD in the UK, which is a cause for concern . Until it is known whether this increasing trend is maintained over time, it is difficult to predict future numbers of cases."

Since 1996, when vCJD was officially linked to eating BSE-infected beef, 69 deaths have been attributed to the brain disease. Six people currently have vCJD. Because so little is known about the incubation period, experts do not know how many people are likely to die from the disease. Estimates range from less than 150 to hundreds of thousands .

In the new study, the raw monthly figures were broken down. With help from victims' relatives, the team determined the date of onset of the disease for each patient, and then grouped data about the start of the disease and deaths into yearly and quarterly categories.

The results, published in the Lancet, suggested an uneven but significant rise in death rates and new cases each year. In 1994, eight people developed first symptoms of vCJD, followed by 10 in 1995, 11 in 1996, 14 in 1997, and 16 in both 1998 and 1999.

Deaths have gone from three in 1995, 10 in 1996 and 1997, 18 in 1998 and 14 last year. So far this year, 14 people have died, suggesting that the final total for 2000 will be far higher than in previous years.

Although infected beef has been removed from the food chain, there are fears that a large number of people could be incubating the disease . Early results from tests of tonsil tissue stored in hospitals after tonsilectomies has so far failed to find evidence of a major epidemic.

Last year, statisticians at City University in London suggested that vCJD was at its peak and that the number of new cases would fall. Prof Philip Thomas, from City, has argued that the rise of vCJD is mirroring that of BSE in cattle which peaked in 1992. He has said the disease may have an incubation period of about eight years and that the number of new cases could fall from next year.

03 Aug 00 - CJD - UK deaths from variant CJD rising by a third each year

A research letter in this weeks issue of THE LANCET states that the incidence of variant Creutzfeldt-Jakob disease (vCJD) has increased by an average of 23% each year since 1994 ; and that death from the disease has increased by about a third each year since 1995 .

The study, by Robert Will and colleagues, analysed the trends in onset and death from vCJD from 1994 to the present. They established dates of vCJD onset by asking relatives of patients about the first appearance of symptoms. To assess whether the incidence of onsets and deaths was increasing over time, they grouped data by year and quarter.

By June 30, 2000, 75 cases of vCJD had been identified by the National CJD Surveillance Unit, 69 of whom had died. 59 cases had been confirmed neuropathologically. The other 16 cases, six of whom were alive, were classified as probable vCJD. The investigators comment that, "given that further cases with onsets in 1999 and 2000 will probably be identified in future months, the number of onsets clearly increases each year". They observe that 14 people have already died from vCJD in the first six months of 2000, compared with 18 deaths for the whole of 1998.

Hester Ward, one of the investigators, comments: "Although absolute numbers remain low, there appears to be a real increase in the incidence of vCJD in the UK, which is a cause for concern . Until it is known whether this increasing trend is maintained over time, it is difficult to predict future numbers of cases"

03 Aug 00 - CJD - Beef exports fail to recover from ban

By Michael McCarthy, Environment Correspondent

Independent ... Thursday 3 August 2000

British beef exports are still only a trickle , a year to the day after the European Union's ban was lifted, the Meat and Livestock Commission (MLC) revealed yesterday.

In the year since Brussels lifted its import prohibition, 500 tons of British beef, worth £5m, have been exported to the Continent. This contrasts sharply with the 274,000 tons worth £520m exported the year before the ban in March 1996.

One of the main reasons, says the MLC, is the stringent regulation of the date-based export scheme insisted upon as the readmission price for British beef to European Union markets. This demands a life history for every animal sent abroad; it also prescribes that abattoirs wanting to export dedicate themselves entirely to exports. So far, only two plants, at Truro in Cornwall and East Kilbride in Scotland, have found this economically worthwhile.

Other hindrances include the high level of the pound against the euro, which makes exports dearer, and the continuing French ban on British beef which, although being challenged, sends an unhelpful signal to other markets.

British beef exports have had to be rebuilt from scratch . An MLC spokesman said. "It's one thing convincing supermarket buyers and even agriculture ministers in various countries, but we also have to convince the consumers ."

The MLC would like the date-based export scheme relaxed so slaughterhouses could devote some days to exports and some to home markets. "The conditions are too onerous," the spokesman said.

02 Aug 00 - CJD - Dentists told of CJD risk in surgery

James Meikle

Guardian ... Wednesday 2 August 2000

Government scientists yesterday warned there was a theoretical risk that the human form of BSE could be spread by infected instruments used in dental surgery .

They have already begun to study nerve tissues from the mouths of victims of bovine spongiform encephalopathy to assess whether these people carry the abnormal protein thought most likely to cause the disease.

The Department of Health has been asked to add an assessment of the risks caused by contaminated dental instruments to a wider survey of surgical equipment. A report this year will look at whether there is a need for expensive changes to procedures in operations and whether more instruments should be only used once rather than sterilised.

The scientists on the committee advising ministers on issues relating to BSE and its human equivalent, vCJD, stress that "there are no grounds for recommending changes to procedures involving dentistry". Present procedures do not completely remove all traces of the infective agent . But James Ironside, a committee member who works at the CJD surveillance unit in Edinburgh, said that conventional sterilisation, if done properly, could "achieve a substantial reduction in the levels of infectivity".

Dr Ironside said there was no evidence (UK Editor;s note: how many times have we heard this familiar phrase?) from comparative studies of vCJD victims that their dental treatment had differed from that of others. The nerves giving sensation to the mouth and gums were however closely linked to the brain, an area of high infectiveness .

There are about 25,000 dentists in the UK and the British Dental Association said there was already government guidance on procedures for patients in high risk groups. These methods might not be practical in high street surgeries and it advised members to refer high risk patients to a hospital or a clinic.

Opticians have already been told not to re-use contact lenses in patient tests, and disposable instruments are increasingly used in surgery . Instruments used on vCJD patients are routinely destroyed .">

02 Aug 00 - CJD - Dentists told to sterilise equipment in BSE warning

By Valerie Elliott, Consumer Editor

Times ... Wednesday 2 August 2000

Dentists are being told to sterilise all surgical instruments in case they are a factor in helping to spread the human form of "mad cow" disease .

The alert was signalled yesterday after the Government's leading advisers on bovine spongiform encephalopathy (BSE) said that they believed there was "a theoretical risk" that the fatal human version of the brain disease, variant Creutzfeldt-Jakob disease, could be passed on by dirty equipment .

Professor Peter Smith, acting chairman of the Spongiform Encepalopathy Advisory Committee (SEAC), said yesterday that dentists must ensure that they followed recommended cleansing practices of their instruments, which involves washing and sterilising to reduce the risk of contamination.

The committee has previously issued warnings about the need to sterilise surgical instruments, but this is the first time that they have issued a warning to dentists.

Professor Smith made clear that he was not recommending the disposal of all dental instruments after their use on a single patient. He admitted that scientists did not know if there was any link between dentistry and cases of vCJD but that they were not prepared to take any chances (UK Editor's comment: if this was true instruments would not be reused).

They were concerned, however, because dental treatment is an invasive procedure and the instruments could be in contact with blood.

He also said that the areas of the mouth anaesthetised in dental work contained cranium nerves that linked to the central nervous system in the brain.

SEAC has also ordered further research on oral tissues of vCJD victims to identify whether infection was present.

Dr James Ironside, a neuropathologist at the CJD surveillance unit in Edinburgh, said that he would now routinely ask relatives of victims if he could carry out post-mortem tests on these tissues. He said: "We will try to get this consent. We are very grateful to relatives for their support and encouragement with post-mortem examinations to allow us to perform this vital research. But people reserve the right to refuse and anything that damages and involves the face in a post-mortem is a sensitive area."

He said that in cases of sporadic CJD there had been no evidence that there was any infectivity in tissue linked to dentistry. He now hoped to conduct the same analysis on vCJD victims.

A spokesman for the Department of Health said that dentists had already been told to take great care in the sterilisation of their instruments because of the risk of Aids as well as possible links to vCJD. Officials would decide in the coming days whether to issue new guidance, he said.

The British Dental Association said that it was fully aware of the risks and that dentists knew that they had to sterilise instruments thoroughly.

The Department of Health is still conducting a review on whether surgical instruments used in operations should be disposed of after use . Surgeons are drawing up a list of the operations that are considered to be most at risk for the possible contraction of vCJD.

Replacement of all instruments after use would cost millions and is not considered necessary. Professor Smith said yesterday, however, that scientists and doctors were looking particularly at possible disposal of instruments used in neurosurgery, the brain and central nervous system.

Professor Smith also yesterday updated the number of cases of vCJD.

There were now 69 deaths and a further eight probable cases, he said, one more than disclosed two weeks ago.

The illness lasted on average some 14 months from first symptoms to death, but some people had died within six months, and the longest suffering had been 39 months.

02 Aug 00 - CJD - Warning for dentists on 'mad cow disease'

By David Brown, Agriculture Editor

Telegraph ... Wednesday 2 August 2000

Dentists were urged yesterday to make sure that their instruments are scrupulously washed and sterilised to help prevent the human equivalent of mad cow disease being spread from patient to patient .

The Spongiform Encephalopathies Advisory Committee [SEAC], the independent team of scientists which advises the Government on new variant Creutzfeldt-Jakob disease (vCJD) in people and bovine spongiform encephalopathy (BSE) in cattle, admitted that it did not know the level of risk of catching the disease in the dentist's chair.

Asked how much of a risk dentistry posed, Prof Peter Smith, acting chairman of SEAC, said: "We do not know." But he said that there were currently no grounds for recommending changes to dentistry procedures.

He acknowledged that washing and sterilising instruments in auto-claves would not destroy all contamination by the deadly vCJD agent but said that the proper hygiene procedures would remove much of it. "What SEAC is saying is that procedures for cleaning and sterilising instruments should be followed. They may be completely followed, maybe not."

So far 69 people have died from vCJD in Britain and another eight "probable" cases are still alive. The victims have ranged in age from 15 to 54 . The committee said it was "significant" that the vCJD trend rose by between 20 to 30 per cent in the first half of this year to 14 cases but said it was too early to forecast accurately the ultimate size of the epidemic. The committee recommended further research.

The Department of Health is currently carrying out an assessment of the risks of spreading all forms of CJD. Its findings are expected by the end of this year. SEAC had previously urged opticians to tighten procedures to prevent vCJD being spread to clients on re-usable contact lenses.

Proposals for the Government's national scheme to eradicate scrapie (UK Editor's note: the FDA in the US announced a 'state of emergency' to eradicate scrapie some time ago), the BSE equivalent in sheep, from flocks in the United Kingdom were released for consultation by the Ministry of Agriculture yesterday.

Under the scheme the taxpayer will pay to test rams for genetic resistance to scrapie, to check for traces of scrapie on farms and will introduce programmes to help producers eliminate the disease from their flocks. But the taxpayer will not have to pay to cull rams which fail the tests or for tests on ewes for genetic resistance.

The Government is anxious to eliminate scrapie from the national flock in case BSE has spread to sheep . So far there is no foolprof test to differentiate between scrapie and BSE in sheep even though the two disease are readily identifiable in the laboratory.

02 Aug 00 - CJD - Beef exports still reeling from mad cow ban

Staff Reporter

Evening Standard ... Wednesday 2 August 2000

British beef exporters have won back less than one per cent of their trade in the year since the EU lifted its ban.Britain has exported only 500 tons of beef in the last year compared with 274,000 tons - worth £520 million - in 1995, the last year before the ban was imposed in the midst of the "mad cow" scare.

A strict export scheme (DBES) now regulates the quality of beef for sale in foreign markets but the high value of Sterling against the Euro in undermining exports

02 Aug 00 - CJD - New Ulster beef trade threatens British sales

By David Brown, Agriculture Editor

Telegraph ... Wednesday 2 August 2000

British farmers will lose sales of live cattle to Northern Ireland worth more than £3.7 million a year under Government plans to persuade Brussels to lift restrictions on beef exports from Ulster.

Meat hygiene and safety controls on shipments of beef and beef products from England , Scotland and Wales to Northern Ireland will also be tightened in a move which could make much of the trade uneconomic .

The proposed new measures, aimed at reinforcing the safety of Ulster meat, were disclosed yesterday in a consultation document issued by Brid Rogers, the farm minister for Northern Ireland, on proposals to allow the resumption of exports of Northern Ireland beef from cattle aged more than 30 months.

Yesterday marked the first anniversary of the Brussels decision to lift its worldwide ban on exports of British beef. In the past year only about 500 tons of beef, worth £5 million , have been sent abroad - a fraction of the trade prior to the eruption of the beef crisis in March 1996 when BSE was linked to a new form of fatal brain illness in people. But the embargo remains on all beef from cattle more than 30 months old produced in the United Kingdom. Exports of live cattle are also banned.

Farmers in Northern Ireland claim they should be treated as a special case because there have been very few incidents of BSE in Ulster. They also say they should be treated no differently when trading with Europe to farmers in the Republic of Ireland who have escaped similar restrictions even though cases of the fatal cattle disease have been increasing there.

Under the scheme, farmers in Ulster would also be able to resume exports of live cattle which are also forbidden from the United Kingdom under EU restrictions to prevent the spread of mad cow disease. David Byrne, EU Commissioner for Health and Consumer Protection, has let it be known that he is keen to remove the over-30 month restriction from Northern Ireland.

But under the plans, no exports of Ulster beef would be allowed from cattle born in Britain or from the offspring of British-born cattle which have been sent there. Such cattle would have to remain in Northern Ireland. The document acknowledges that producers in Britain would suffer financial losses from the plans and would have to seek alternative markets for their cattle.

But the beef industry in Ulster would gain by between £35 million and £40 million a year if it could cash in on the full export opportunities for its beef and beef products. The document says: "Relaxation of controls on exports resulting from Northern Ireland's low BSE incidence will set a useful precedent for Great Britain when it achieves similar incidence."

Nick Brown, Minister of Agriculture, said: "The Government has been exploring informally with the European Commission how Northern Ireland could benefit from its low rate of BSE.

"I am pleased to say that we have now developed proposals which the commission have indicated might provide the basis for a decision. These would permit the export of live cattle, beef and bovine products from Northern Ireland without the restrictions which currently apply in the UK ."

The National Farmers' Union of England said yesterday that it welcomed moves to remove restrictions on beef exports from Northern Ireland but it gave a warning that it would oppose any move to restrict cattle sales to Ulster.

A spokesman said: "We cannot have a deal at any price. We and the beef trade will oppose anything that disrupts existing trade between Britain and Northern Ireland." The United Kingdom beef industry has been given until October 6 to respond to the plans.

01 Aug 00 - CJD - Mad Sheep

Wilson Ring

AP US & World ... Tuesday 1 August 2000

Montpelier, Vt. (AP) -- A federal judge ruled Tuesday that two flocks of sheep the federal government says might suffer from a version of the always-fatal mad cow disease should be killed. U.S. District Judge J. Garvan Murtha in Brattleboro refused to issue an injunction that would have stopped the slaughter of the flocks, which are owned by Larry and Linda Faillace of East Warren, and Houghton Freeman of Stowe. The Faillaces and Freeman argued the tests used by the federal government to condemn their sheep were inconclusive.

"When preventing diseases with lengthy incubation periods... the USDA cannot be expected to wait until clinical signs of disease appear," the judge wrote, "because any actions implemented at that time would be taken years too late." The judge said the owners have until Aug. 7 to appeal.

Thomas Amidon, a lawyer representing Freeman, said an appeal is possible. "We are looking at what are options were. We haven't made any decisions yet," he said. "Obviously we knew that we had a huge burden to overcome going in," Amidon said. "But we really had to have someone look at it. To that extent we are appreciative to the fact that a third party -- the judiciary -- has taken a look at it."

Last month Agriculture Secretary Dan Glickman ordered 376 sheep in the two flocks to be destroyed after tests on the carcasses of four animals from Freeman's farm were found infected with a transmissible brain disease that could be bovine spongiform encephalopathy, the scientific name of mad cow disease. The owners of 21 sheep in a third flock voluntarily sold their sheep to the USDA to be destroyed before Glickman's order.

mad cow disease devastated the cattle industry in Great Britain in 1995. It has killed about 70 people in Europe and -- because the incubation period of the disease can be up to 10 years -- the number of human victims is expected to rise.

Even though scientists aren't sure the four Vermont sheep carcasses had a form of mad cow disease, the USDA says it's better to destroy them and avoid any possibility the disease could gain a foothold in North America.

The sheep in all three flocks were either imported from Belgium in 1996 or are the offspring of imported sheep. The USDA feels the sheep might have eaten contaminated feed in Europe before they were brought to Vermont.

01 Aug 00 - CJD - EU experts assess risk of mad sheep disease

David Evans

Reuters World Report ... Tuesday 1 August 2000

European Union scientists are probing the nightmare possibility of an epidemic of mad cow disease in the sheep population.

"It's an area we are concerned about and there is a scientific risk assessment programme under way," Beate Gminder, spokeswoman for EU Health Commissioner David Byrne, told Reuters on Tuesday. "But so far," she stressed, "there is no scientific proof."

Professor Emmanuel Vanopdenbosch, chairman of the working group in the European Commission on mad cow disease, or BSE, told Belgian newspaper De Morgen, "The BSE question with sheep is a timebomb that continues to tick."

Although the cattle brain-wasting disorder blamed for causing more than 50 human deaths has yet to show up in the sheep population, a disease of the same family, scrapie, is widespread. Scrapie is not believed to be dangerous to humans, but EU scientists believe sheep could contract the cattle variant. If BSE (bovine spongiform encephalopathy) took hold, it could spread quickly from flock to flock and need drastic measures to contain it.

A sheep's relatively small size means the disease would affect nervous tissue much quicker than with cattle and the rate of infection would be much higher. Vanopdenbosch said many more animals would have to be culled than in the costly mad cow crisis. "It can be expected that the disease would spread much faster and on a much larger scale than with cattle," he said.

"Not only Europe but also the rest of the world could be in trouble if it turns out that the 'mad sheep phenomenon' does exist. Sheep have been exported to all corners of the world for years," Vanopdenbosch said.

The prospect of the disease turning up in the sheep population has been highlighted by a case in the United States. Three flocks in Vermont have been found to contain animals with a neurological ailment that could be mad cow disease. Some animals of Belgian origin there have tested positive for TSE (transmissable spongiform encephalopathy), the family of diseases that includes BSE and scrapie.

U.S. agriculture officials have ordered the sheep to be destroyed although it could be years before they determine exactly which type of TSE the animals were suffering from.

Spokeswoman Gminder said the European Commission had been in contact with U.S. officials over the Vermont case, and had requested tissue samples for their own investigation.